Aneurysmal aorto-left ventricular tunnel causing right ventricular outflow tract obstruction, associated with bicuspid aortic valve.

We report the case of a newborn with an aneurysmal aorto-left ventricular tunnel causing significant paravalvular aortic regurgitation and obstruction of the right ventricular outflow tract (RVOT), coexisting with a bicuspid aortic valve. The coexistence of the two malformations together with the obstruction of the RVOT is very rare. In this case, the prompt diagnosis and surgery led to significant improvement of the clinical status and to recovery of the left ventricular function (increase of the ejection fraction from 21 to 41 %), underlining the importance of early diagnosis in this rare malformation.